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Refractory Hypoxemia in a 23-Year-Old Patient With Budd-Chiari SyndromeBicaval Syndrome

Jeroen J. H. Bunge, MD; Ubbo S. Wiersema, MD; Adriaan Moelker, MD, PhD; Jasper van Bommel, MD, PhD; Eric T. T. L. Tjwa, MD, PhD
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From the Department of Intensive Care (Drs Bunge and van Bommel), the Department of Gastroenterology and Hepatology (Drs Wiersema and Tjwa), and the Department of Radiology (Dr Moelker), Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands.

CORRESPONDENCE TO: Eric T. T. L. Tjwa, MD, PhD, Department of Gastroenterology and Hepatology, Erasmus Medical Centre University Hospital, Room Hs-312, PO Box 2040, 3000 CA Rotterdam, The Netherlands; e-mail: etjwa@erasmusmc.nl


Drs Bunge and Wiersema contributed equally to this work.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;146(5):e149-e152. doi:10.1378/chest.13-2879
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Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state, leading to arterial and venous thrombosis. We present a 23-year-old patient, suspected of having Budd-Chiari syndrome due to antiphospholipid syndrome, who developed severe and progressive hypoxemia, requiring prolonged mechanical ventilation. After a detailed but unsuccessful workup, a contrast CT scan revealed an occluded superior vena cava and azygos vein-superior vena cava junction and massive right-to-left shunting through a network of systemic to pulmonary venous collaterals. Restoring normal blood flow from the azygos vein into the right atrium by stenting the azygos-superior vena cava junction resolved the hypoxemia immediately. Within the same procedure, the hepatic outflow obstruction was successfully treated by stenting a severe stenosis of the suprahepatic inferior vena cava caused by calcified thrombus.

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